Atrioventricular Valve Incompetence Research Articles

Post-transplant left atrial function depends on the technique used for orthotopic heart transplantation

Abstract Background Even short episodes of post-transplant atrial fibrillation (AF) have been shown to increase the risk of adverse outcomes, including thromboembolic events, graft failure, and increased mortality. Of the two most commonly used heart transplantation techniques - bicaval and total orthotopic heart transplantation (OHTx), the former approach combines the donor and recipient atria, and may predispose to supraventricular arrhythmias, especially atrial fibrillation, as well as lead to a higher incidence of atrioventricular valve incompetence. Existing data relate mainly to the more non-physiological left atrial (LA) size and geometry after bicaval OHTx, while little is known about differences in LA function between patients treated with both techniques. Aim To compare LA functional and morphological characteristics between patients after bicaval and total OHTx. Methods The study group consisted of 28 patients who underwent OHTx between May 2021 and December 2022: 14 consecutive patients after total OHTx and 14 subjects after bicaval OHTx matched by age and time from heart transplant. Echocardiographic examination included standard measurements and left ventricle (LV) and LA longitudinal deformation assessment by speckle tracking technique. Results All enrollees were male. There were no differences between the groups after total and bicaval OHTx in anthropometric and laboratory parameters. Routine endomyocardial biopsies performed during follow-up after OHTx in all patients did not show severe graft rejection. No significant differences were found in LV dimensions, ejection fraction and stroke volume, Doppler velocities of mitral inflow and mitral annulus, as well as LV global longitudinal strain. In contrast to LV measures, significant between-group differences were demonstrated for LA parameters: LA volume index was significantly higher, and peak atrial longitudinal strain (PALS) corresponding to LA early diastole in the reservoir phase and peak atrial contraction strain (PACS) corresponding to LA systole were significantly higher in the total OHTx group than in the bicaval OHTx group (Table). Conclusions Total OHTx technique is associated with a more favorable LA morphology and function, which may explain the lower prevalence of atrial fibrillation reported in the literature in patients treated with this method. The clinical significance of reduced LA contribution to LV filling in patients subjected to bicaval OHTx needs further exploration.

Endomyocardial fibrosis in Sudan: clinical and echocardiographic features.

SummaryObjective:Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan.Methods:The study started in January 2007 and is on-going. All the patients attending our echocardiography clinics in four different hospitals in Khartoum, Sudan, were included. Transthoracic echocardiography was used as the main diagnostic and selection tool. The diagnosis of EMF was based on predefined criteria and definitions, and was further supported by additional clinical, ECG, laboratory and chest X-ray findings.Results:Out of 4 332 cases studied, 23 (0.5%) were found to have features of EMF. Females constituted 52% and the age range was 24 to 67 years. All patients presented with dyspnoea grades III–IV. Advanced heart failure with gross fluid overload was seen in 54% of cases and ascites was seen in 30%. EMF was biventricular in 53%, left ventricular in 29% and right ventricular in 18% of cases. Apical and ventricular wall fibrosis was found in all cases, followed by atrial enlargement, atrioventricular valve incompetence, ventricular cavity obliteration, restrictive flow pattern and pericardial effusion. Additional echocardiographic features are defined and discussed.Conclusion:Although a rare disease, cases of EMF can be identified in Sudan if a high index of suspicion is observed. New echocardiographic features of ventricular wall layering, endocardial fibrous shelf and endomyocardiopericarial fibrosis were identified and are discussed.

Edge-to-edge Alfieri technique for mitral valve repair

To outline the current indications to the edge-to-edge technique for mitral valve repair. Indeed, after its introduction in the early 1990s, criteria for appropriate patient selection and technical issues of this approach have been progressively refined over the years and are now rather standardized. The long-term results (up to 17 years) of this approach in the setting of degenerative mitral regurgitation with bileaflet and anterior leaflet prolapse have been recently reported and confirm the important role of this method in the surgical armamentarium of mitral valve reconstruction. In addition, recent data support the use of this method in new special situations and settings (systolic anterior motion prevention/treatment, congenital atrioventricular valve incompetence, as a 'rescue' procedure in case of suboptimal conventional repair). In mitral regurgitation competence of the mitral valve can be restored by using a 'functional' rather than a conventional 'anatomical' repair. This 'functional' repair is realized by means of the edge-to-edge technique that consists in suturing together the facing portions of the anterior and posterior mitral valve leaflets in correspondence to the location of the regurgitant jet. The edge-to-edge technique was introduced in the early 1990s and has provided a useful contribution to the surgical armamentarium of mitral valve repair. Appropriate indications and awareness of the important technical aspects of the procedure are prerequisites for a good outcome. In this review the present role of the edge-to-edge repair, particularly in terms of indications and contraindications, will be outlined and discussed.

Evaluation of the Aristotle complexity models in adult patients with congenital heart disease†

The adult congenital heart disease (CHD) population has surpassed the paediatric CHD population. Half of all mortality caused by CHD occurs in adulthood; in some patients, it occurs during surgery. We sought to assess the potential risk factors for adverse outcome after cardiac operations in adults with CHD, and to evaluate the predictive power of the Aristotle score models for hospital mortality. Procedure-dependent and independent factors, as well as the outcome factors of all consecutive patients aged 16 or more who underwent surgery for CHD between 2005 and 2008 at our institution were evaluated according to the European Association for Cardio-Thoracic Surgery Congenital Database nomenclature. An Aristotle basic complexity (ABC) and an Aristotle comprehensive complexity (ACC) score were assigned to each operation. The discriminatory power of the scores was assessed using the area under the receiver operating characteristics (AuROC) curve. During 542 operations, 773 procedures were performed. The early mortality rate was 2.4%, and the early complication rate was 53.7%. Tricuspid valve replacement (P = 0.009), mitral valve replacement (P < 0.001), elevated lung resistances (P = 0.002), hypothyroidism (P = 0.002) and redosternotomy (P = 0.003) emerged as risk factors for 30-day mortality. Tricuspid valve replacement (P < 0.001), tricuspid valvuloplasty (P = 0.006), mitral valve replacement (P = 0.003), shunt implantation (P = 0.009), surgical ablation (P = 0.024), myocardial dysfunction (P = 0.014), elevated lung resistances (P = 0.004), hypothyroidism (P = 0.002) and redosternotomy (P < 0.001) emerged as risk factors for complications. Mean ABC and ACC scores were 6.6 ± 2.3, and 9.0 ± 3.7, respectively. The AuROCs of the ABC and the ACC scores for 30-day mortality were 0.663 (P = 0.044), and 0.755 (P = 0.002), respectively. The AuROCs of the ABC and the ACC scores for complications were 0.634 (P < 0.001), and 0.670 (P < 0.001), respectively. Surgery for adults with CHD can be performed with low early mortality. However, complications are frequent, especially in patients who require repeat operations for atrioventricular valve incompetence. The ACC score may be helpful to estimate the risk of early mortality.

Re-repair of the left atrioventricular valve in atrioventricular septal defects: the morphologic approach to the role of Gore-tex band reduction annuloplasty

Incompetence of the left atrioventricular valve remains the final Achilles' heel of repair of atrioventricular septal defects (AVSDs), despite low operative mortality in the modern era. We have analysed the morphological basis for valvar incompetence in our own series of repairs, and in doing so, reviewed the morphology of the annulus in AVSDs, before and after surgical repair. We reviewed retrospectively re-repair operations of the left atrioventricular valve following previous repair of AVSDs at the Great Ormond Street Hospital from 1 January 1994 to 31 December 2007. From this case series, the reasons for valvar incompetence were identified and techniques used for re-repair recorded. We also incorporated the detailed morphology of the annulus of the left atrioventricular valve before and after repair from our cardiac morphology archive. Thirty-three patients had undergone re-repair of the left atrioventricular valve between 1 January 1994 and 31 December 2007. Twenty patients underwent re-repair of the left atrioventricular valve within 1 year of initial repair, and 13 cases beyond 1 year after repair. Cases re-repaired within 1 year mainly did so as a result of leaflet tears or valvar dysplasia. Cases re-repaired after this time mainly had multiple areas of valvar leakage, including central incompetence. Two patients underwent a second re-repair, which were dealt with by a partial ring from a thin-wall Gore-Tex graft for reduction annuloplasty. Out of the 121 cardiac morphologic specimens, 53 had undergone previous complete repair. Following repair, the annulus of the new left atrioventricular valve was composed of artificial patch material on its septal portion. Left atrioventricular valve incompetence following previous repair usually involves repair of the zone of apposition between the left bridging leaflets. Many of these valves also require annuloplasty to attain competence. Given the shape, growth potential and morphologic composition of the annulus in these cases, we have performed some of these repairs with a partial flexible ring from a thin-walled 3.5-mm Gore-Tex graft as a reduction annuloplasty that can be shaped to fit the exact contours of this annulus.

Staged total cavopulmonary connection for complex congenital heart diseases

To review the experience of staged total cavopulmonary connection (TCPC) in complex congenital heart diseases. From June 1998 to March 2008, 22 patients underwent staged TCPC for complex congenital heart diseases. Among them, 9 were univentricular and pulmonary artery valve stenosis; 3 were univentricular and pulmonary artery atresia; 1 was transposition of great arteries, crisscross heart and pulmonary artery valve stenosis; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery atresia and atrioventricular valvular regurgitation; 1 was complete atrioventricular canal defects, left ventricular hypoplasia, pulmonary artery valve stenosis and atrioventricular valvular regurgitation after Glenn procedure; 1 was mirror image dextrocardia, single ventricle, pulmonary artery atresia, major aortopulmonary collateral arteries (MAPCAs) and right pulmonary arteriovenous fistula after Glenn procedure; 4 were tricuspid atresia and pulmonary artery valve stenosis; 1 was tricuspid atresia and pulmonary atresia; 1 was mirror image dextrocardia, double-outlet of right ventricle, left ventricular hypoplasia, pulmonary artery valve stenosis, tricuspid incompetence, and MAPCAs. Among them, 5 patients received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC. Seventeen patients received bidirectional Glenn procedure, the mean age was (5.9+/-4.4) years old. Pulmonary artery pressure pre-Glenn procedure was 17 to 20 mm Hg (1 mm Hg=0.133 kPa). Atrioventricular valve incompetence in 3 patients. Nakata index was less than 200 mm2/m2 in 4 patients before the first stage operation. The age of TCPC procedure was (9.6+/-4.9) years old, the interval time was (3.7+/-1.2) years. There was one in-hospital death, the mortality was 4.5%. The patient with univentricular and pulmonary atresia, received systemic-to-pulmonary artery shunt, bidirectional Glenn procedure and TCPC and died of pneumorrhagia. Other patients were recovered well, postoperative central venous pressure was 12 to 18 mm Hg, percutaneous oxygen saturation was 90% to 96%. The cardiac function were in NYHA class I to II. The staged TCPC was a good procedure in high-risk Fontan candidates. The results were satisfactory for those patients. This staged strategy may extend the operative indications for the Fontan procedure.

Pitfalls in Diagnosis and Clinical, Echocardiographic, and Hemodynamic Findings in Endomyocardial Fibrosis: A 25-Year Experience

Endomyocardial fibrosis (EMF) is a fascinating disease entity of unknown etiology. It is prevalent in the tropical zone. Its essential features are the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of the inflow tract and apex of one or both ventricles. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary muscle involvement, and progressive reduction of the cavity of the involved ventricle leading to restriction in filling and atrial enlargement. This article will present 21 patients with EMF who were initially referred to our hospital from 1979 to 2004 with different diagnoses: rheumatic heart disease with mitral and or tricuspid regurgitation (n = 9), constrictive pericarditis (n = 6), restrictive cardiomyopathy (n = 1), hypertrophic cardiomyopathy apical type (n = 2), dilated cardiomyopathy (n = 2), and Ebstein malfunction of the tricuspid valve (n = 1). The clinical, echocardiographic, hemodynamic, and angiographic findings in these 21 patients are presented; echocardiographic findings lead to the right diagnosis. The presence of a small ventricle with obliteration of the apex and large atrium shown on two-dimensional echocardiography is highly suggestive of EMF. Endomyocardial fibrosis (EMF) is a fascinating disease entity of unknown etiology. It is prevalent in the tropical zone. Its essential features are the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of the inflow tract and apex of one or both ventricles. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary muscle involvement, and progressive reduction of the cavity of the involved ventricle leading to restriction in filling and atrial enlargement. This article will present 21 patients with EMF who were initially referred to our hospital from 1979 to 2004 with different diagnoses: rheumatic heart disease with mitral and or tricuspid regurgitation (n = 9), constrictive pericarditis (n = 6), restrictive cardiomyopathy (n = 1), hypertrophic cardiomyopathy apical type (n = 2), dilated cardiomyopathy (n = 2), and Ebstein malfunction of the tricuspid valve (n = 1). The clinical, echocardiographic, hemodynamic, and angiographic findings in these 21 patients are presented; echocardiographic findings lead to the right diagnosis. The presence of a small ventricle with obliteration of the apex and large atrium shown on two-dimensional echocardiography is highly suggestive of EMF.

Catheter ablation of supraventricular tachycardia in a child with situs ambiguus, single ventricle, interrupted IVC and Glenn shunt

A 13 year old boy with drug refractory Supraventricular Tachycardia (SVT) was admitted for electrophysiological study and possible radiofrequency catheter ablation. He was a known case of complex congenital cyanotic heart disease: dextrocardia, common atrium, single ventricle, common atrioventricular (AV) valve with atrio ventricular septal defect and AV valve incompetence. He underwent Modified Blalock Taussig shunt in 1995 followed by Glenn Shunt in 1998. He had recurrent SVT despite amiodarone, digoxin and diltiazem.

Surgical therapy for the univentricular heart.

The natural history of the univentricular physiology is determined primarily by the presence (severity) or absence of obstruction to pulmonary blood flow and secondarily by the single ventricular cardiomyopathy that develops in response to chronic volume overload. Important obstruction to pulmonary blood flow will affect survival because of severe hypoxia and may necessitate surgical treatment (systemic to pulmonary artery shunt or cavopulmonary shunt depending on the age of the patient and a host of other factors). In the absence of significant obstruction to pulmonary blood flow, the symptomatology is one of congestive heart failure with later development of irreversible pulmonary vascular disease. In this situation, a pulmonary artery band is indicated to limit pulmonary blood flow and reduce pulmonary artery pressures to acceptable levels. Even in the presence of significant limitation of pulmonary blood flow, the single ventricle is a volume-loaded ventricle as it receives both pulmonary and systemic venous return. Chronic volume overload and persistent hypoxia is detrimental to ventricular function and gradually lead to the development of atrioventricular valve incompetence. Complete separation of oxygenated and deoxygenated blood may be obtained by ventricular septation in suitable cases (beyond the scope of this presentation) or by systemic venous diversion to the pulmonary (the Fontan circulation) arteries thus achieving complete oxygenation simultaneous with abolition of any recirculation.

Left atrioventricular valve repair technique in partial atrioventricular septal defects

Background. The aim of our study was to evaluate the effect of chordal transfer around the cleft on left atrioventricular valve competence in the late postoperative period.Methods. Forty-four adult patients underwent surgical correction of partial atrioventricular septal defect between 1983 and 1997. Fenestration was found in 8 patients (18.2%) and cleft, in 35 (79.5%). There was no chordal support of the free edges of the left superior and left inferior leaflets around the cleft in 18 patients. Two chordae were mobilized from the left lateral leaflet and reimplanted into the tip of the left superior and left inferior leaflets around the cleft.Results. At 5 years postoperatively, left atrioventricular valve insufficiency was severe in 5 patients and moderate in 11 patients who had had cleft closure alone. In contrast, severe valvular insufficiency was present in only 1 patient in the group with chordal transfer (p < 0.05). Reoperation was done in 5 patients with isolated cleft closure. Left AV valve replacement was performed in 1 patient.Conclusions. Chordal transfer plus cleft closure with interrupted sutures significantly reduces early and late left atrioventricular valve incompetence and also decreases the rate of reoperation.

Atrioventricular discordance: Results of repair in 127 patients

Objective: The conventional management of patients with atrioventricular discordance is directed at associated lesions, taking advantage of physiologic “correction”; however, the morphologic right ventricle and tricuspid valve support the systemic circulation. Questions surrounding survival using this approach led us to analyze our institutional results. Methods: All patients with atrioventricular discordance undergoing biventricular repair were analyzed (n = 127, 1959-1997), excluding those with functionally univentricular hearts. The ventriculoarterial connection associated with atrioventricular discordance varied and was most commonly discordant (87%), but occasionally concordant (6%), double-outlet right ventricle (6%), or double-outlet left ventricle (1%). At initial presentation, the most common lesions associated with atrioventricular discordance were ventricular septal defect (86%), pulmonary stenosis (64%), tricuspid regurgitation (28%), and atrioventricular block (12%). Nine patients underwent a double switch procedure to create ventriculoarterial concordance and the remainder were managed conventionally without correcting discordant connections. Results: Operative mortality was 6% and did not vary by associated lesion. Twenty years after repair, survival was 48%. Within 20 years, 56% of patients required reoperation, usually for atrioventricular valve incompetence (n = 16), pulmonary stenosis (n = 16), or both (n = 3). Pacemakers were required in 50 patients, 4 before repair, 40 within 2 months of repair, and 6 remotely after repair. In early follow-up, the double switch procedure (n = 9) had equivalent mortality and a high pacemaker requirement for atrioventricular block. Conclusions: Analysis of conventional management of atrioventricular discordance revealed cumulative increases in mortality, systemic atrioventricular valve (tricuspid) replacement, complete atrioventricular block, and incidence of reoperation. Alternative management should be examined. (J Thorac Cardiovasc Surg 1999;117:1190-203)

Clinical Results of the Staged Fontan Procedure in High-Risk Patients

Background. For high-risk Fontan candidates, the introduction of a bidirectional Glenn shunt before total cavopulmonary connection (a two-staged strategy) may extend the indications for the Fontan procedure. The clinical results of the two-staged and one-staged Fontan procedure were thus reviewed and compared. Methods. Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (≥20 mm Hg), high pulmonary vascular resistance (≥3 Wood units), small pulmonary artery (Nakata index <200 mm 2/m 2), atrioventricular valve incompetence (≥ moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same pe-riod, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group). Results. In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group). Conclusions. A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.

Results of left atrioventricular valve reconstruction after previous correction of atrioventricular septal defects.

The objective of this study was to determine causes of severe left atrioventricular (AV) incompetence and the factors leading to the success of valve repair later after correction of atrioventricular septal defects (AVSD). A total of 28 patients aged 5 months to 38 years (mean age 6.7 years) were operated for significant (grade II-III) left AV valve incompetence (LAVVI), two months to twenty-five years (median 1.5 years) after correction of complete (11 patients) or partial atrioventricular septal defects. Fourteen patients had initially undergone surgery during infancy. At reoperation a completely open or partially sutured cleft was found in 16 patients combined with dysplastic valve tissue in four cases, with a fibrotic valve in three cases, with posterior leaflet prolapse in two cases, with a double orifice valve in three cases, and a parachute valve in two cases. Partial or complete reopening of a previously sutured cleft caused by suture dehiscence was found in 12 cases combined with a fibrotic valve in five cases, with a dysplastic valve in one case and with severe deformity of valve in one case. A combination of these anomalies was observed in seven patients in both groups. Left atrioventricular valve repair including cleft closure combined with annuloplasty and other surgical procedures resulted in the disappearance or significant diminishing of LAVI in 18 patients (64%). Severe SAVI persisted in six patients, five of them exhibiting a combination of several additional left AV valve anomalies (fibrotic or dysplastic valve, parachute valve). Five of these six patients underwent successful left AV valve replacement with a mechanical prosthesis 7 days to 2 years after reoperation. The presence of additional left AV valve anomalies was the single statistically significant factor for recurrent major LAVVI after reoperation (P = 0.0106). There were two postoperative deaths in patients with mild LAVVI after surgery, and no late deaths. An open cleft is the major factor of late severe SAVVI after correction of AVSD. Although suturing the cleft in conjunction with performing annuloplasty improved valvular function in most of the cases, the presence of severe left AV valve anomalies increased the risk of recurrent LAVVI and the need for valve replacement, thus playing a major role in determining the outcome of valve reconstruction in patients after reoperation.

Etiological factors influencing the development of atrioventricular valve incompetence after heart transplantation

Etiological factors influencing the development of atrioventricular valve incompetence after heart transplantation

Venous blood flow pattern suggesting tachycardia-induced 'cardiomyopathy' in the fetus.

Studies in the fetal lamb and human fetus demonstrated that, in the late stage of supraventricular tachycardia, myocardial dysfunction (tachycardia-induced 'cardiomyopathy') may appear and even persist after drug-induced conversion to sinus rhythm. The objective of this study was to verify whether these changes in cardiac function are reflected in the venous system. In seven fetuses with supraventricular tachycardia (five with hydrops and four of these with atrioventricular valve incompetence during tachycardia) between 24 and 33 weeks of gestation, spectral Doppler analysis of flow velocity waveforms in the inferior vena cava and the ductus venosus was performed before and after drug-induced cardioversion, and was compared with reference values known from the literature and our own indices obtained in 129 normal pregnancies. After drug-induced conversion from supraventricular tachycardia to sinus rhythm associated with a change from a pulsatile to a normal biphasic forward venous blood flow pattern, abnormal venous indices resulting from increased reverse blood flow during atrial contraction persisted for 2-42 days (median 12 days). Severe functional tricuspid insufficiency can lead to a significant decrease in the systolic peak velocity with deterioration of the venous blood flow indices. In the first days after supraventricular tachycardia, rapid changes in these indices seem to depend predominantly on the decrease of tricuspid insufficiency and the increase of the systolic peak velocity. In addition to the persistence of atrioventricular valve regurgitation, abnormal indices of venous blood flow during sinus rhythm indicate the existence of altered myocardial function, suggesting reversible tachycardia-induced 'cardiomyopathy'. Blood flow indices in the venous system seem to reflect changing myocardial function very sensitively and rapidly and, therefore, appear very useful in the surveillance of the fetus with tachyarrhythmia under drug therapy, especially with regard to the assessment of cardiac function at the time of cardioversion and in the period after supraventricular tachycardia.

Modified fontan operation in functionally univentricular hearts: Preoperative risk factors and intermediate results

Background: The modified Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy. To eliminate the influence of different surgical strategies, only patients undergoing total cavopulmonary anastomosis were included in this retrospective study. Methods: Seventy-two patients had been operated on at ages ranging from 7 to 219 months, with 29 patients younger than 4 years. Twenty-three patients had mean pulmonary artery pressures higher than 15 mm Hg, elevated pulmonary arteriolar resistances (>3 U · m 2), or elevated end-diastolic ventricular pressure (>12 mm Hg). Associated systemic or pulmonary venous anomalies were present in 22 patients (30%), and atrioventricular valve incompetence was present in 21 patients (29%). Results: The overall mortality rate was 9.7% (7/72). Variables with significant effects on postoperative mortality were associated systemic or pulmonary venous anomalies, atrioventricular valve incompetence, mean pulmonary artery pressure greater than 15 mm Hg, and prolonged cardiopulmonary bypass time. Postoperative morbidity resulted mainly from atrial arrhythmia (20%). Clinical signs of protein-losing enteropathy or atrial thrombi were rare (3% and 6%, respectively). Postoperative hemodynamic data from 48 surviving patients (74%) revealed a mean transpulmonary gradient of 6.3 mm Hg, systemic venous pressure greater than 12 mm Hg in only 10 patients, and cardiac index less than 3 L · min -1 · m -2 in 18 patients. Conclusion: In a high proportion of hearts with complex univentricular anatomy, associated anomalies, and borderline hemodynamics, the Fontan operation can be performed as a total cavopulmonary anastomosis with acceptable intermediate postoperative morbidity and hemodynamic results. (J T HORAC C ARDIOVASC S URG 1996;112:658-64)

Ten-year experience with surgical treatment of partial atrioventricular septal defect: Risk factors in the early postoperative period

Partial atrioventricular septal defects are electively repaired with good results. However, recent reports suggest that such repair is potentially a high-risk surgical procedure. Our aim was to determine the risk factors of adverse outcome early after surgical treatment of atrioventricular septal defects in our hospital. A retrospective study was done in 100 consecutive patients from 2 months to 50.6 years old (median 3.6 years) who underwent surgical correction between January 1984 and December 1993. An intermediate form of the lesion was noted in 31% of cases. Congestive heart failure occurred in 50% of cases. Preoperative left atrioventricular valve incompetence (moderate to severe) was present in 63% of patients. Severe abnormalities of left subvalvular apparatus were noted in 28% of patients. The cleft of the left atrioventricular valve was closed in 76% of cases. The study was done to determine risk factors associated with hospital mortality (13%), postoperative residual left atrioventricular valve incompetence (23%), and early reoperation (14%) within the first 30 postoperative days. Univariate analysis showed that age at the date of operation and cleft closure were not related to an early adverse outcome. A stepwise logistic regression with variables selected by univariate analysis identified infections and severe abnormalities of left subvalvular apparatus as predictive factors of early death (odds ratios, 28.07 and 6.18, respectively), preoperative left atrioventricular valve regurgitation as a predictive factor of residual postoperative left atrioventricular valve regurgitation (odds ratio, 5.34), and severe abnormalities of left subvalvular apparatus as a predictive factor of early reoperation (odds ratio, 5.27). These results emphasize the importance of the severity of the morphologic features of the left subvalvular apparatus, the occurrence of early postoperative infections, and the presence of residual left atrioventricular valve regurgitation as risk factors in the early period after surgical correction of partial atrioventricular septal defects. (J T horac C ardiovasc S urg 1996;112:14-20)

Surgical Repair of Corrected Transposition of the Great Arteries with Cardiac Anomalies: A Report of Fifty-Three Cases

Between February 1964 and June 1994, we operated on 53 patients with corrected transposition of the great arteries associated with cardiac anomalies. Their ages ranged from 2.5 to 37 years (mean 15.9 years). There were 36 patients with type SLL and 17 with type IDD. Forty-three patients had ventricular septal defect with pulmonary stenosis, 7 had VSD with pulmonary hypertension and only 3 patients had atrioventricular valve incompetence without ventricular septal defect. Atrial septal defects were found in 13 cases and patent ductus arteriosus in 2. The main operative procedures were closure of ventricular septal defect (49), closure of atrial septal defect (13), resection of pulmonary stenosis (43) and pulmonary annulus enlargement (3). Additional procedures were bypass between the morphological left ventricle and the pulmonary artery using valved external conduit (4), tricuspid valve repair (4), tricuspid valve replacement (4) and one Fontan operation. Nine patients died (17%) within 30 days of operation. The mortality rate decreased from 29.2% to 6.9% after 1988. The main cause of early death was low cardiac output syndrome. The most common perioperative complications were complete heart block (5) and residual tricuspid valve incompetence (4). Forty of the 44 survivors were followed up from 2 months to 5 years. There were 2 late deaths due to tricuspid incompetence. Our surgical experience in the prevention of operative complications are discussed.

Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves

The surgical management of patients with double-outlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a subject of controversy. Biventricular repair has theoretic advantages because it establishes normal anatomy and physiology. In some instances, however, it seems to carry too high operative risk, and a univentricular heart repair is preferred. Since 1984, we have operated on 34 patients with double-outlet right ventricle (n = 15) or transposition of the great arteries (n = 19) with isolated straddling tricuspid valve (n = 17), isolated straddling mitral valve (n = 9), both mitral and tricuspid straddling (n = 2), or abnormal insertion of tricuspid (n = 7) or mitral (n = 2) chordae in the left ventricular outlet, precluding an adequate tunnel construction. Straddling was categorized according to the location of the papillary muscle insertion in the opposite ventricular chamber: type A, on the edge of the ventricular septal defect (n = 14); type B, on the opposite side of the ventricular septum away from the edge of the defect (n = 8); type C, on the free wall of the opposite ventricular chamber (n = 8). Abnormal chordal insertions were classified according to the location of their attachments around the edges of the defect. Three types of chordal distribution were identified: on the aortic conus, on the pulmonary conus crossing the ventricular septal defect, or around the defect closing it like a curtain. All but three patients had two ventricles of adequate size. Sixteen patients underwent palliation. Median age at the definitive operation was 6.5 months (range 1 to 130 months). Thirty patients underwent a biventricular repair and four had a univentricular repair. Biventricular repair was achieved by an arterial switch operation in 18 patients and by tunnel construction from the left ventricle to the aorta in 12. In isolated straddling of types A and B, the ventricular septal defect was closed by adjusting the septal patch on the ventricular side above the straddled papillary muscle. In type C, the patch was sewn over the papillary muscle by applying it on the septum. In double straddling, the ventricular septum was incised between the two papillary muscles, and an ellipsoid patch was used to reconstruct the septal defect, directing each subvalvular apparatus into its own ventricular chamber. When the abnormal chordae in the left outflow tract inserted on the aortic or pulmonary conus, the conus was incised and tailored to make a flap, leaving an unobstructed left ventricular outflow tract. In two patients the subvalvular apparatus was resected and reattached to the patch. Curtainlike chordae were a contraindication to biventricular repair in double-outlet right ventricle but not in transposition. There were four early deaths and one late death, all occurring in the group having biventricular repair. Death was due to myocardial ischemia (n = 1), right ventricular hypoplasia (n = 1), pulmonary hypertension (n = 1), and residual subaortic stenosis (n = 1). Two patients had moderate to severe postoperative atrioventricular valve incompetence, caused by a cleft in the mitral valve in one patient. Three patents were reoperated on for subaortic stenosis (n = 1), pulmonary stenosis (n = 1), and mitral regurgitation (n = 1). Mean follow-up of 30.7 ± 19.4 months was achieved in the survivors. All but one patient (univentricular repair) were in New York Heart Association class I, without atrioventricular valve incompetence. Actuarial survival at 4 years was 85.3% ± 3%. We conclude that straddling or abnormal distribution of chordae tendineae of the atrioventricular valves does not preclude biventricular repair in double-outlet right ventricle or transposition of the great arteries provided that the ventricles are of adequate size. Curtainlike abnormal tricuspid chordae remain a contraindication to biventricular repair in double-outlet right ventricle. (J T horac C ardiovasc S urg 1996;111:527-35)

Bidirectional cavopulmonary shunt in patients with multiple risk factors

Twenty-five patients in whom a modified Fontan operation was deferred because of multiple risk factors underwent a bidirectional cavopulmonary shunt. Two or more of the following risk factors were present in all: age less than 1 year, severe pulmonary artery distortion, impaired left ventricular function, subaortic obstruction, anomalous systemic-pulmonary venous connection, atrioventricular valve incompetence, and increased mean pulmonary artery pressure. Additional procedures included take-down of systemic-pulmonary artery shunt, atrial septectomy, pulmonary artery reconstruction, bulboventricular foramen enlargement, and atrioventricular valve repair. There were three hospital deaths (12%). Mean follow-up is 21 months. There was no late mortality. Mean oxygen saturation increased from 71% to 83%. Results obtained from pulmonary artery reconstruction, enlargement of bulboventricular foramen, and atrioventricular valve repair were satisfactory for the patients in whom these risk factors were present preoperatively. Ventricular function also improved in the survivors in whom it was previously deteriorated, this being related to the suppression of the sources of ventricular volume overload. In this risk group of patients for a modified Fontan operation, a bidirectional cavopulmonary shunt provided adequate palliation at reasonable low risk. Early bidirectional cavopulmonary shunt would minimize complications originating from systemic-pulmonary shunts such as pulmonary artery distortion and the potential harm of chronic ventricular volume overload.